Morgan Phillips, shown here with her husband, Stephen, had to wait four days to meet Jude in the NICU as they both recovered, but their connection was instant. (Photo credit: Phillips family)
As the Marketing & Communications Specialist for the Department of Obstetrics and Gynecology at UT Southwestern Medical Center, Morgan Phillips had long looked forward to growing her own family. She and her husband, Stephen Phillips, were thrilled to find out they were expecting in late October 2023. The pregnancy was everything she’d dreamed of, Morgan said, uneventful and easy – until she hit 32 weeks.
What happened over the next several weeks was beyond unlikely – a 1 in 10 million chance, according to Stephen, who spent many hours researching the conditions. And it would challenge the collective expertise of teams across UT Southwestern and Children’s Health to unravel a medical mystery that put the lives of Morgan and her son in critical danger.
‘This was never part of our plan’
At a regular check-in with her obstetrician, Morgan received the unsettling news that her blood pressure was elevated. A reading at home the next week was so high Morgan was admitted to UTSW’s William P. Clements Jr. University Hospital for monitoring with suspected preeclampsia, a pregnancy-related condition that causes high blood pressure and high amounts of protein in urine.
As her blood pressure continued to rise even with treatment, doctors told Morgan they would almost certainly need to deliver her baby early, prompting a surge of anxiety.
“This was never part of our plan,” Morgan said. “Our nursery wasn’t ready, we had no car seat with us, and I started spiraling.”
After four days of monitoring in the hospital, Morgan awoke on a Sunday morning – the day after her pregnancy reached 34 weeks. She felt fine, but as the day progressed, more troubling symptoms arose. An insidious pain developed in her upper abdomen that steadily grew worse, making her struggle to breathe and writhe in pain.
Jamie Morgan, M.D., was the maternal-fetal medicine specialist at UT Southwestern for Morgan Phillips.
Bloodwork performed every six hours since she’d been admitted had been normal, but with Morgan’s rapidly rising pain her maternal fetal medicine (MFM) specialist, Jamie Morgan, M.D., requested new labs right away. When the initial numbers came back, they were so abnormal the lab had to run them again. But they were right. And somewhat shocking, said Dr. Morgan.
“I’d never seen anything like this before.”
A life-threatening complication
Within just a few hours, Morgan’s liver enzymes had jumped from the tens to the thousands. Her platelets had dipped dangerously low, and her red blood cells were breaking down. Together, these findings were unmistakable for HELLP syndrome, a severe form of preeclampsia and a life-threatening complication affecting just 0.6% of pregnancies.
HELLP stands for hemolysis (red blood cell degradation), elevated liver enzymes (a sign of poor liver function), and low platelets (a loss of the blood component responsible for clotting). This confluence of conditions meant that Morgan’s baby needed to be born as soon as possible – the only effective treatment for HELLP.
This is Jude's first photo at Clements University Hospital’s NICU at UT Southwestern. (Photo credit: Phillips family)
Morgan was prepped for an emergency cesarean section, necessitating general anesthesia. Unable to join his wife in the operating room, Stephen paced in Morgan’s labor and delivery room. “I was there for what felt like ages – it was very stressful,” he said.
Catherine Spong, M.D., Chair of Obstetrics and Gynecology at UTSW, migrated between the operating room and the labor and delivery room where Stephen was waiting, sharing with him what she knew. “I wanted to keep him updated,” she said. “Things happened so rapidly, and there was so much going on.”
When Morgan and Stephen’s son, Jude, was born, he was gray and needed immediate resuscitation. His mother’s dire condition, his prematurity, and his exposure to general anesthetics had taken a toll.
Once he was breathing, Jude was whisked to the neonatal intensive care unit (NICU) for care, and Dr. Spong took Stephen to meet Jude. During his stay in the NICU, nurses drew a single drop of blood from Jude’s heel for newborn screening, a panel of tests for congenital diseases given to every newborn in Texas. Meanwhile, Morgan was wheeled to the intensive care unit (ICU), where she’d spend the next three days.
A novel NICU experience
LaKeitha Foster Truehill, M.D., was Jude's neonatologist.
While she recovered in the ICU, Morgan was too sick to meet Jude. Her critical care physicians and obstetricians treated the consequences of her HELLP syndrome, requiring multiple blood transfusions and monitoring the organs affected by her ordeal, particularly her kidneys, which had largely stopped functioning.
Jude stayed in the NICU, learning to eat and gradually gaining weight, said LaKeitha Foster Truehill, M.D., Jude’s neonatologist. Stephen visited Jude multiple times a day and attended conferences with Jude’s entire care team – a unique approach at Clements University Hospital’s NICU that allows parents to ask questions and gain a deeper understanding of their child’s condition.
“This is the only hospital I have worked in where parents are invited into the conference rooms where rounds are held with the NICU team and able to listen in to the care plan, ask questions, and have their questions answered as well,” Dr. Foster Truehill said. “We have physicians, bedside nurses, pharmacists, and dietitians present, and rounds are held daily, even on the weekends. It’s very hands on.”
Four days after his birth, Morgan was strong enough to visit Jude in the NICU.
“I was nervous to meet him, nervous that I wouldn’t feel connected to him because we didn’t have that experience of him being placed on my chest as soon as he was born,” Morgan said. “I worried that we’d missed out on so much critical time for bonding, but we didn’t.”
Morgan cried when she finally held Jude for the first time, and his bedside nurse told her that she’d never seem him that calm and thriving.
A genetic curveball
Stephen Phillips took Jude to his first appointment with a genetic specialist. (Photo credit: Phillips family)
Morgan and Stephen continued to hear good news from the NICU – Jude was steadily gaining weight and doing well, despite the difficult circumstances surrounding his birth. But they also received another piece of news they weren’t expecting: Jude’s newborn screen was positive for a rare condition called X-linked adrenoleukodystrophy (X-ALD).
This genetic condition, passed from mothers to sons, causes an inability to break down very long-chain fatty acids. Over time, the disease gradually damages the fatty sheath that insulates nerves in the brain and spinal cord, impairing nervous system function. X-ALD also affects the adrenal glands, which make hormones that regulate the immune system, blood pressure, and other critical functions.
Although treatments exist for X-ALD – including bone marrow transplantation and gene therapy, which work better the earlier this disease is diagnosed – the news was especially difficult for Morgan and Stephen to receive after her battle with HELLP syndrome. Stephen, vice president at a Fort Worth-based biotechnology venture capital firm, calculated that the likelihood that a family would have the combined burden of gestational hypertension, preeclampsia, HELLP, and X-ALD was nearly 0.00001%.
“We found out about the possibility of Jude’s diagnosis while both of us were still hospitalized,” Morgan said. “It’s hard. We never thought this was what our story was going to be.”
As soon as Jude’s care team received his results, they brought in a new member: Luis Umaña, M.D., a pediatric geneticist who specializes in inborn errors of metabolism and rare genetic conditions including X-ALD that affect how the body processes food into energy.
Luis Umaña, M.D., was Jude's pediatric geneticist.
Dr. Umaña explained that Children’s Medical Center Dallas, where most UTSW pediatric faculty treat patients, is the major referral center in North Texas for these diseases, with a team of experts in X-ALD including pediatric endocrinologists, pediatric neurologists, and bone marrow transplant and gene therapy experts. Patients with this rare condition, affecting just one in 14,000-17,000 people, can receive care at Children’s and UTSW over their entire lives.
“We are one of the few places in which patients have continuity of care from childhood to adulthood,” Dr. Umaña said.
Connecting the dots
After further testing confirmed Jude’s preliminary diagnosis, Morgan tested positive as a carrier for X-ALD. Recent research suggests carriers can also have some effects from the condition. Suspecting that being an X-ALD carrier may have played a role in Morgan’s HELLP syndrome, Dr. Morgan referred her case to Gary Cunningham, M.D., Professor and Chair Emeritus of Obstetrics and Gynecology at UTSW and chief author/editor of Williams Obstetrics, the definitive textbook read by nearly every obstetrics and gynecology physician trainee in the U.S.
Every Thursday morning, UTSW’s maternal fetal medicine fellows and physicians meet with Dr. Cunningham to discuss their most challenging and unusual cases. After Dr. Morgan presented Morgan’s case at this weekly conference, he confirmed that her X-ALD carrier status and HELLP syndrome were probably linked.
Jude overcame a difficult start in life and is now a "smiley and giggly and chatty" boy, said his proud mom. (Photo credit: Phillips family)
“The research suggests that carriers for this condition may have more manifestations than we’ve previously appreciated, which almost certainly contributed to Morgan’s extreme symptoms. There’s no way to definitively prove [this connection], but we have a strong suspicion,” Dr. Morgan said.
These days, Morgan continues to recuperate from her ordeal. Her kidneys have largely recovered, but she sees nephrologists at UTSW to monitor their function. Other members of her family are currently being tested to see if they are also carriers for X-ALD. Now 6 months old, Jude continues to grow strong with no outward symptoms of his X-ALD. A dedicated team will introduce therapies if and when he becomes symptomatic, Morgan said.
She added that Jude’s early diagnosis and multidisciplinary care, as well as the expertise that saved both their lives, aren’t available at most hospitals; their story may have been very different, she said, if she and Stephen had driven instead to the closest hospital to their home.
“I’m so fortunate that this was the experience I had because if I had been somewhere else, I truly don’t feel like we would have had this outcome. I always had so much peace and reassurance because I was so confident with the hands I was in,” Morgan said. “Now we have Jude, and he is so smiley and giggly and chatty, and he makes us laugh every day. We are so extraordinarily lucky.”
(Photo credit: Adeline Merrick)